Catamenial haemothorax

Haemothorax is a collection of blood with haematocrit >50% in the space between the visceral and parietal pleura. Although traumatic or iatrogenic in a vast majority of cases, it may rarely be spontaneous. Spontaneous haemothorax (SH) is most commonly due to pneumothorax but may be related to malignancy, vascular causes or coagulopathy besides various other aetiologies.[] Thoracic endometriosis (TE) is a rare cause of SH and should be highly suspected in females of reproductive age, especially with pelvic endometriosis or history of pelvic surgery.[] Thoracic endometriosis syndrome (TES) is the term given when endometrial tissue occurs within the thoracic cavity. The most common clinical presentations of TES include chest pain, cough and dyspnoea. TES can manifest as one of the following entities: catamenial pneumothorax (73% of cases), catamenial haemothorax (CH, 14% of cases), haemoptysis (7% of cases) or lung nodules (6% of cases).[] Clinical manifestations are defined as catamenial when they occur between 72 h before and 72 h after the onset of menses.[] CH is an extremely rare manifestation of TES that is usually mild and self-limiting. We report a rare case of a young woman who presented with massive CH. CASE REPORT A 22-year-old unmarried female referred to the CVTS department from the department of obstetrics (OBG) and gynaecology in view of recurrent right-sided haemothorax related to menstruation. The patient had a history of cough and dyspnoea at the time of menstrual cycle for 1 year. She visited a local hospital for the same and ultrasonography of the abdomen showed haemoperitoneum and computed tomography (CT) thorax showed right-sided haemothorax for which right tube thoracostomy was done. OBG consultation was done and advised medical amenorrhoea for 2 months. She was discharged after 5 days. The patient had similar complaints after 4 months during her menses, for which she again consulted the same hospital and X-ray showed right-sided haemothorax, for which right tube thoracostomy was done and drained 1 L of haemorrhagic pleural fluid. She was admitted for 1 week. Chest tube was removed and the patient was referred to OBG Department, Medical College, Kozhikode. She was managed with blood transfusion and supportive measures. She was provisionally diagnosed as a case of pelvic endometriosis with associated thoracic endometriosis. She was advised medical management with oral contraceptives and shifted to the CVTS department for further evaluation. She was a known case of Bernard–Soulier syndrome, diagnosed at the age of 16 years. Menarche was attained at the age of 15 years. Since then, the cycles were regular with normal bleeding, not associated with dysmenorrhea. On clinical examination, her vitals were stable, and air entry was reduced on the right side. Magnetic resonance imaging (MRI) thorax showed moderate free fluid in the right hemi-thorax and peritoneal cavity and a suspicious focal altered signal intensity area in the parietal pleura overlying dome of the right hemi-diaphragm showing minimal peripheral blooming-endometriotic deposits and bilateral cystic lesions in the ovary. All investigations for a malignant or infectious aetiology were unremarkable. The patient was submitted to the right posterolateral thoracotomy after getting haematology fitness and transfusion of platelets. Thoracotomy revealed 3 cm × 3 cm suspicious endometriotic deposit over the dome of the right hemi-diaphragm and 2 cm × 2 cm lesion over diaphragmatic surface of the right lower lobe. Excision of the lesion and pleurodesis were done. The peroperative and investigative findings of the patient are given in [Figures 1234]. Pleural biopsy showed evidence of old and recent haemorrhage with focal areas of stromal decidualisation, compatible with endometriosis. She underwent aggressive deep breathing exercises and incentive spirometry post-operatively. She was discharged home with hormonal therapy. At follow-up, chest imaging showed no recurrence of pleural effusion.

Endometriosis is a benign condition in which endometrial stroma and glands are found outside the uterus. The mean age at diagnosis varies from 25 to 35 years and the most common affected areas are the uterosacral ligaments, rectouterine and vesicouterine excavations, uterine tubes, sigmoid colon and round ligaments. However, it may rarely occur in extrapelvic locations such as the umbilicus, abdominal scars, breasts, extremities, pleural cavity and the lungs.[] Pleural endometriosis is a rare form of endometriosis, and its incidence among general population is still unknown. Thoracic endometriosis may involve either or both the pleura and the lung parenchyma. Pleural endometriosis manifests with chest pain and dyspnoea as catamenial pneumothorax or CH. Parenchymal endometriosis, on the other hand, usually presents with chest pain, dyspnoea and haemoptysis. Women of reproductive age, especially those in their mid-30s, are most commonly affected.[] Our patient's presentation was with cough and shortness of breath during dysmenorrhoea. A massive right-sided pleural effusion and a grossly bloody aspirate were relatively straightforward for thoracic endometriosis. Although the pathogenesis of pulmonary endometriosis is not well understood, three main theories have been hypothesised. Sampson theorised that menstrual blood with endometrial fragments could regurgitate from the Fallopian tube into the peritoneal cavity. This blood could find its way into the sub-phrenic space and pass through the diaphragmatic fenestrations into the pleural cavity[] Ivanoff theorised that irritant blood with endometrial fragments could pass through pleural fenestrations and produce metaplasia of the pleural surface, which is histologically similar to that of the peritoneum[] Others theorised that obstetrical and gynaecological procedures that disrupt endometrial blood vessels and lymphatics allow lymphovascular entry of endometrial tissue causing parenchymal disease. This theory is supported by studies that find an association between pulmonary endometriosis and certain forms of endometrial trauma.[] The radiological abnormalities in TE are transient; diagnostic yield is increased when performed around menses. Chest X-ray and CT images are usually the initial studies to detect pneumothorax and haemothorax; however, MRI is more sensitive in revealing the endometrial lesions than CT scan. CA-125 may be elevated in the seroma and pleural fluid but is not specific, and likewise, pleural fluid cytology is seldom of any help. Classically, to diagnose TE, the identification of both endometrial stroma and glands is required on histological examination; it is nevertheless considered suggestive if stroma alone or pulmonary parenchymal haemorrhages or haemosiderin laden macrophages are detected.[] In clinical practice, it is hard to detect intact glands and stroma with hormone receptor study because of autolysis and degradation of proliferative ectopic endometrial tissue 48 h after menstruation.[] Only one-third of the cases of TE have typical histopathological finding.[] Hence, in many cases, the diagnosis is based on a compatible clinical picture, suggestive imaging studies and a bloody pleural aspirate, corroborated with characteristic findings on Video Assisted Thoracoscopic Surgery (VATS)/thoracotomy. In our case, the patient had recurrent massive right-sided haemothorax, and right posterolateral thoracotomy showed endometriotic deposit over the dome of the right diaphragm and diaphragmatic surface of the right lung. The biopsy of lesions revealed stromal decidualisation but no glands. TE treatment includes medical and surgical modalities which can be combined.[] Medical approach (ideal for women who wish to preserve fertility) depends on suppression of ovarian oestrogen secretion by oral contraceptives, progesterone agonists, gonadotropin-releasing hormone agonists or danazol for at least 6–12 months.[] However, the recurrence rate is more than 50% on stopping the treatment.[] Surgery is considered in patients with refractory or recurrent disease.[] Best surgical modality is VATS, which allows removal of ectopic endometrial tissue, pleurectomy and closure of diaphragmatic defects.[] Pleurodesis is an alternative or can be considered additionally. In our case, we did excision of endometriotic deposits over the diaphragm, pleura and lung through right posterolateral thoracotomy and advised to continue oral contraceptives.

TE-associated CH is a rare aetiology for SH. It usually involves the right side. Endometriosis should be considered as a differential diagnosis of pleural effusion in females of reproductive age, especially when the symptoms are exacerbated by menses. The successful treatment of TE is also as challenging as is the diagnosis. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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